Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac anomaly characterized by hypoplastic or even absent pulmonary valve, to-and-fro flow across the pulmonary valve annulus, and dilatation of main pulmonary artery and branches. It is crucial to evaluate the degree of dilatation of pulmonary arteries and the presence of associated malformation and chromosomal anomalies affecting pregnancy decision. We described two- and three-dimensional (3D) echocardiographic findings of one fetus with APVS and indicated the beneficial contribution of 3D technology in understanding the anatomy.
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